Eur J Pediatr Surg 2008; 18(6): 410-414
DOI: 10.1055/s-2008-1038923
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Implication of Unfavorable Histology, MYCN Amplification and Diploidy for Stage I and II Neuroblastomas

A. S. de Buys Roessingh1 , 4 , A.-L. Rougemont2 , C. Wiesenauer1 , S. Barrette3 , D. Bouron-dal Soglio2 , M. Lallier1
  • 1Department of Pediatric Surgery, University Hospital, Hôpital Sainte-Justine, Montréal, Canada
  • 2Department of Pathology, University Hospital, Hôpital Sainte-Justine, Montréal, Canada
  • 3Department of Onco-Hematology, University Hospital, Hôpital Sainte-Justine, Montréal, Canada
  • 4Service de Chirurgie Pédiatrique, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland
Further Information

Publication History

received June 23, 2008

accepted after revision July 7, 2008

Publication Date:
14 November 2008 (online)


Background: Surgery is the first line treatment for low-grade neuroblastomas. In stage I tumors, the presence of MYCN amplification is rarely detected and the Shimada histology is not always taken into consideration when deciding on the treatment. This study concerns the significance of these two factors in the evolution of children with low-grade neuroblastomas. Methods: We analyzed the assessment and follow-up of children with low-grade neuroblastomas (stages I and II) with or without MYCN amplification, with either a favorable or unfavorable histology and with or without tumor cell diploidy. Favorable histology was defined as stroma-poor tumors with more than 5 % differentiating neuroblasts and a mitosis karyorrhexis index (MKI) of less than 100/5000 cells. Results: From 1995 to 2006, out of 114 neuroblastomas, nine (7.9 %) were stage I and 21 (18.4 %) stage II. Of these 30 patients, 27 underwent surgery alone and three received chemotherapy after surgery. The combination of MYCN amplification, unfavorable histology and diploidy was noted in one patient who developed metastases within two months. MYCN amplification alone was noted in two cases who are still tumor-free after two years. Unfavorable histology alone was noted in four patients, of whom one suffered a recurrence of the tumor (previously stage I) and three are tumor-free after six years. Tumor cell diploidy alone was present in 11 patients whose evolution is satisfactory. Conclusion: Because MYCN amplification and unfavorable histology are rare in early stage neuroblastomas, these tumors may be misclassified if they are not investigated further. It seems that no single clinical or biological feature can be considered a significant factor in establishing a prognosis or determining whether additional treatment is required.


Dr. M.D., Ph.D. Anthony S. de Buys Roessingh

Service de Chirurgie Pédiatrique
Centre Hospitalier Universitaire Vaudois (CHUV)

46, Rue du Bugnon

1011 Lausanne